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霍奇金淋巴瘤指南解读,程淑琴,(WHO)HL 分为两种类型,淋巴细胞为主型霍奇金淋巴瘤(LPHL) 经典的霍奇金淋巴瘤(CHL) CHL 分为4 种亚型: 结节硬化型CHL(NSCHL ) 混合细胞型CHL(MCCHL) 淋巴细胞消减型CHL(LDCHL) 富淋巴细胞型CHL(LRCHL)。 LPHL 占HL 病例的5%,CHL 占95%。,病 理,CHL 的特点是在炎性背景下存在R-S 细胞, LPHL 缺乏R-S 细胞,但存在肿瘤性淋巴细胞,有时称为爆米花细胞 LPHL 表现为结节性或弥漫性形式 结节性亚型:表现为大量B 淋巴细胞中出现肿瘤性淋巴细胞 弥漫性亚型:背景细胞主要为T 细胞。,免疫组化,CHL:R-S 细胞大多表达CD15和CD30,而CD3 和CD45 常常阴性 40%以下的病人可检测到CD20 推荐CD3CD15CD20、CD30 和CD45 LPHL :CD45+和CD20+,不表达CD15,极少表达CD30 指南推荐CD3、CD15CD20CD21、CD30 和CD57 染色,经典的霍奇金淋巴瘤,在初始诊断和检查后,病人分为下列几组: I-II 期 III-IV 期 I-II 期的病人,根据是否存在不利因素,进一步分为下列亚组: IA-IIA 期(有利) I-II 期(不利伴巨块型疾病) I-II 期(不利伴非巨块型疾病),Staging and Prognosis,Each stageis subdivided into A and B categories.,“A” indicates that no systemic symptoms are present and “B” is assigned to patients with unexplained weight loss of 10% of their body weight, unexplained fevers, or drenching night sweats.,Patients with HL are usually classified into 3groups:,early-stage favorable (stage I-II with no unfavorable factors);,early-stage unfavorable (stage I-II with any of the unfavorable factors such as large mediastinal adenopathy; 23 nodal sites of disease; B symptoms; extranodal involvement; or significantly elevated erythrocyte sedimentation rate ESR 50),advanced-stage disease (stageIII-IV).,FOLLOW-UP AFTER COMPLETION OF TREATMENT AND MONITORING FOR LATE EFFECTS,CR should be documented including reversion of PET to “negative“ within 3 months following completion of therapy. It is recommended that the patient be provided with a treatment summary at the completion of his/her therapy, including details of radiation therapy, organs at risk, and cumulative anthracycline dosage given. Follow-up with an oncologist is recommended, especially during the first 5 years after treatment to detect recurrence, and then annually due to the risk of late complications including second cancers and cardiovascular disease.kk,ll Late relapse or transformation to large cell lymphoma may occur in NLPHL. The frequency and types of tests may vary depending on clinical circumstances: age and stage at diagnosis, social habits, treatment modality, etc. There are few data to support specific recommendations; these represent the range of practice at NCCN Member Institutions.,Follow-up After Completion of Treatment up to 5 Years,Interim H&P: Every 36 mo for 12 y, then every 612 mo until year 3, then annually Annual influenza vaccine Laboratory studies: CBC, platelets, ESR (if elevated at time of initial diagnosis), chemistry profile as clinically indicated Thyroid-stimulating hormone (TSH) at least annually if RT to neck. Acceptable to obtain a CT scan at 6, 12, and 24 mo following completion of therapy, or as clinically indicated. PET/CT only if last PET was Deauville 4-5, to confirm complete response., Counseling: Reproduction, health habits, psychosocial, cardiovascular, breast self-exam, skin cancer risk, end-oftreatment discussion. Surveillance PET should not be done routinely due to risk for false positives. Management decisions should not be based on PET scan alone; clinical or pathologic correlation is needed,Follow-up and Monitoring After 5 Yearskk,Interim H&P: Annually Annual blood pressure, aggressive management of cardiovascular risk factors Pneumococcal, meningococcal, and H-flu revaccination after 57 y, if patient treated with splenic RT or previous splenectomy (according to CDC recommendations) Annual influenza vaccine Cardiovascular symptoms may emerge at a young age. Consider stress test/echocardiogram at 10-y intervals after treatment is completed. Consider carotid ultrasound at 10-y intervals if neck irradiation. Laboratory studies: CBC, platelets, chemistry profile annually TSH at least annually if RT to neck Biannual lipids Annual fasting glucose,Follow-up and Monitoring After 5 Yearskk,Consider low-dose chest CT for patients at increased risk for lung cancer.mm Annual breast screening: Initiate 810 y post-therapy, or at age 40, whichever comes first, if chest or axillary radiation. The NCCN Hodgkin Lymphoma Guidelines Panel recommends breast MRI in addition to mammography for women who received irradiation to the chest between ages 1030 y, which is consistent with the American Cancer Society (ACS) Guidelines. Consider referral to a breast specialist. Colonoscopy every 10 years for patients age 50, if high risk begin at age 40, which is consistent with ACS Guidelines. Counseling: Reproduction, health habits, psychosocial, cardiovascular, breast self-exam, and skin cancer risk. Treatment summary and consideration of transfer to PCP. Consider a referral to a survivorship clinic.,PRINCIPLES OF SYSTEMIC THERAPY (1 of 2),Classical Hodgkin Lymphoma The most common variants of chemotherapy used at NCCN Member Institutions include ABVD and Stanford V. Routine use of growth factors is not recommended. Leukopenia is not a factor for delay of treatment or reduction of dose intensity (except for escalated BEACOPP).,Regimens,ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) ISRT,Stanford V (doxorubicin, vinblastine, mechlorethamine, etoposide, vincristine, bleomycin, and prednisone)*,Escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone),Escalated BEACOPP followed by ABVD with ISRT,Regimens,Nodular Lymphocyte-Predominant Hodgkin Lymphoma* The most common chemotherapies used at NCCN Member Institutions for NLPHL are listed below.,ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) rituximab,CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) rituximab,CVP (cyclophosphamide, vinblastine, prednisolone) rituximab,Rituximab,Involved-site Radiation Therapy (ISRT),Dose: Combined Modality Therapy Non-bulky disease (stage I-II): 20*30 Gy (if treated with ABVD), 30 Gy (if treated with Stanford V) Non-bulky disease (stage IB-IIB): 30 Gy Bulky disease sites (all stages): 3036 Gy PET scan Deauville 3-4 following chemotherapy: 3045 Gy ISRT Alone (uncommon, except for NLPHL): Involved regions: 3036 Gy (the dose of 30 Gy is mainly used for NLPHL) Uninvolved regions: 2530 Gy,PRINCIPLES OF SYSTEMIC THERAPY FOR RELAPSED OR REFRACTORY DISEASE (1 OF 2)Regimens(listed in alphabetical order, Brentuximab vedotin (only for CHL)1 C-MOPP (cyclophosphamide, vincristine, procarbazine, prednisone) (category 2B) DHAP (dexamethasone, cisplatin, high-dose cytarabine)
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